Obituary for Dr. John C. Steele, 1934–2022

John Steele, who has died at the age of 87, was a neurologist who was devoted to attending his patients and describing and understanding the causes of their maladies. He will be best remembered by his colleagues for his contribution of the seminal description of progressive supranuclear palsy (PSP) and his later investigation of the parkinsonism–dementia/amyotrophic lateral sclerosis complex (lytico-bodig) of Guam. He was born in 1934 in Toronto into a medical family, and he studied medicine at the University of Toronto from 1952 to 1960. He then trained in medicine, neurology, and neuropathology in Toronto from 1960 to 1966. During his residency at Sunnybrook Hospital, he looked after a 62-year-old Ukrainian veteran with progressive gait unsteadiness and a vertical gaze palsy who died in December 1960.1 Neuropathological examination at the Banting Institute identified widespread neurofibrillary degeneration, which was initially diagnosed as postencephalitic parkinsonism. J. Clifford Richardson, attending physician and later head of neurology, had identified his first patient with this as yet unclassified disorder in 1955, and after Jerzy Olszewski reviewed the pathology of 7 similar cases, the trio concluded that this was a distinct “new” clinico-pathological entity. In retrospect, the disorder had probably been reported a few times previously in the literature, but had not yet been defined as a discrete syndrome. Richardson presented the cases at the June 1963 meeting of the American Neurological Association following which the neuropathologist Asao Hirano stood up and drew attention to the clinical and pathological similarities of the disorder and the parkinsonism dementia complex of Guam. John Steele led the writing of the 9 cases published in April 1964 in Archives of Neurology and subsequently suggested in a single-authored article published in Brain in 1972 that PSP was a relatively common condition.2-4 Steele-Richardson-Olszewski syndrome was a popular alternative label for the disease for many years, but the classical presentation later came to be known as Richardson's syndrome, a change in nomenclature strongly supported by Steele. After his neurology training in Canada, Steele spent time at the National Hospital Queen Square working with Dr. Denis Williams and then studied clinical neurophysiology in Marseille in 1966 and 1967 and spent a further period of training at the Prasat Neurological Hospital in Bangkok. He then returned to Toronto where he worked as a pediatric neurologist at the Hospital for Sick Children, Toronto, publishing on migraine, infectious syndromes, and alternating hemiplegia of childhood. Working in Thailand had whetted his appetite for adventure and exploration, and after a year working in Bristol, England, he moved to the “vast wilderness” of the Pacific Ocean, taking up a position as a staff physician and neurologist based at the Majuro Hospital in the Marshall Islands. He recalled an idyllic island life as an “oceanic neurologist” working with the islanders in delivering health care across the Marshall Islands. He returned to London to complete a Masters degree program at the London School of Hygiene and Tropical Medicine in 1977 and then returned to Pohnpei in the Eastern Caroline Islands, Micronesia, as chief of staff and director of education. In later years, he told friends that he had chosen a different path to a conventional academic teaching hospital career, choosing a neurological life of exploration and discovery in the Pacific. By 1983, he had moved a further 1000 miles northeast to Guam, which was to be his home for the next 30 years. In the 1950s, Kurland and Mulder5 described a geographic isolate of amyotrophic lateral sclerosis (ALS) and the associated parkinsonism–dementia complex (known locally as lytico-bodig). On Guam, ALS was a 100 times more common than in North America or Western Europe. When Steele arrived on Guam, he was surprised to discover that patients with lytico-bodig had overlapping clinical features with PSP, with axial rigidity, a gait and balance disorder, and in some a vertical gaze palsy. A nomadic neurological journey that had started at the Sunnybrook Hospital in Toronto had ended up 20 years later, 7500 miles away in the Western Pacific. Steele lived for a long time in Umatac village, the epicenter of lytico-bodig on the southern part of the island, and kept meticulous clinical records of his patients. His early collaborations with well-planned National Institutes of Health neuro-epidemiological studies on Guam did not end well in part because he preferred a narrative to a questionnaire-based approach, and his integration in the local community was the polar opposite to research teams flying in and out of remote locations to collect data and biosamples (“helicopter science”). By the early 1990s, he was working as an independent academic clinician on Guam where he established an international network of friends and collaborators who were attracted by the mystery of lytico-bodig. Many of them visited him in Guam and worked and published with him on the disease. They include Bak, Ben-Shlomo, Calne, Caparros-Lefebvre, Cox, Farrer, Gwinn, Hanlon, Hardy, Keck, Lee, Lees, Lepore, McGeer, Miklossy, Morris, Perez-Tur, Sacks, Spencer, and Wresch. Many hoped that lytico-bodig would become the Rosetta stone of neurology—if the cause of this high-prevalence focus could be identified on Guam, then this surely would lead to an understanding of the causes of neurodegeneration in other parts of the world. However, studies of diet, calcium/aluminum metabolism, genetics, local cultural practices, and cycad exposure all proved inconclusive. The most secure evidence defined by his research related to the rapidly declining prevalence of lytico-bodig in people born after 1949 and the common co-occurrence of linear retinal pigment epitheliopathy, which appeared to be consistent with a retinal larval infection in some but not all patients with lytico-bodig. These observations together with neuropathological studies led to the conclusion that lytico-bodig was a multiprotein neurodegenerative disease possibly related to an infectious agent that has now disappeared from Guam. Lytico-bodig may never be definitively explained.6, 7 Steele could be by turns charismatic, single-minded, and assertive, and he always held the welfare of the patients under his care close to his heart. His research sprang from a desire to understand the nature of their diseases. He was a commanding and dynamic speaker at international meetings lecturing on lytico-bodig and the history of PSP. He was also a persuasive and influential advocate for the Lytico-Bodig Association and the PSP charities, Cure PSP and the PSP Association. His talks were always carefully prepared, eloquent, and classical in form. Despite illness and recurrent treatments in the last few years of his life, he remained active, traveling around the world and commenting and communicating on lytico-bodig. He was also tireless in his efforts to encourage young researchers to study lytico-bodig and PSP. He had 3 children and 3 grandchildren, and following his late retirement, he spent part of his time with his family in California and Bellingham, Washington. His last months were spent in Bali, where he died surrounded by his children. On his request, his ashes were scattered in the ocean off the coast of Candidasa, on the southeast part of the island. We are grateful for the advice and help of Julia Steele, his daughter, in preparing this obituary.

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